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Xiong et al. Nature of liver lymphoma

in which these lesions are defined as primary hepatic place specifically of cancer. [24]
lymphoma and described as a malignancy that is very
rare and frequently misdiagnosed, but has a rather Some authors stated that primary hepatic lymphoma
better prognosis by the combination of surgery and should be differentiated from hepatitis, hepatic
chemotherapy as the treatment than primary hepatic metastasis, primary hepatic tumors and secondary
carcinoma. [15-17] This paper discusses several aspects hepatic lymphoma, however, they attempted to prove
of the entity of primary hepatic lymphoma, especially that a liver lymphoma was primary even when bone
the dilemmas in diagnosis. marrow or portal lymph nodes were involved and
interpreted that this involvement was metastasis but
CLASSIFICATION OF LYMPHOMA not infiltration. [25,26]

In 1965, Ata et al. reported, for the first time, a case Secondary hepatic lymphomas are defined by some
[18]
of primary reticulum cell sarcoma of the liver. Later authors as liver lymphomas with extra hepatic foci of
in 1971, Torres et al. described primary reticulum lymphoma and found at the first onset or afterwards,
[19]
cell sarcoma of liver in cancer. Since then, cases or systemic lymphoma with secondary hepatic
and case series have been accumulated reporting involvement. This description is also conflicting with
the characteristics of primary hepatic lymphoma and the above definition of primary lymphoma. [27]
stating that this disease is extremely rare with the
absence of lymphoproliferative disease outside the The WHO classification of lymphoma is the generally
liver. [20,21] accepted interpretation. In this system, lymphomas
are classified by the normal cell type that looks most
Some authors defined primary hepatic lymphoma as like the tumor and interpreting cytogenetic, molecular
a very rare malignant tumor with the features of liver or phenotypic features. The three main groups are
[28]
involvement and without involvement of other organs the T cell, B cell, and natural killer cell lymphomas.
and tissues including bone marrow, lymph nodes, the Less common groups are identified and listed in the
spleen and peripheral blood until at least 6 months subtypes. It has been debated that this classification
after diagnosis. [14,22] However, Caccamo’s criteria needs validating in a large series of patients before
which many authors have cited for the diagnosis of publication. [2,3] Interestingly in this classification, the
primary hepatic lymphoma were proposed in 1986 term “primary” is used but not specified, like primary
and based on the data of a single patient who was effusion lymphoma, primary central nervous system
complicated with liver cirrhosis, Kaposi’s sarcoma, lymphoma, etc., and a number of subtypes are
and involvement of gastric mucosa and abdominal described as “provisional entities” [Table 1].
lymph nodes. [22,23] In many of the case reports, the
patient follow-up was not long enough as “at least 6 ESTABLISHMENT OF DIAGNOSIS
months after diagnosis” and some diagnoses were
determined just after the biopsy even when there Clinical manifestations usually suggest diseases
were enlarged retroperitoneal lymph nodes and bone involving the liver but not the lymphatic and immune
marrow infiltration by lymphoma. [11,14,15] system. [29,30] Many cases are diffuse large B cell
lymphoma and the patients show B-symptomatology
Is there indeed a disease entity named primary hepatic of weight loss, fever, and night sweats, as well as
lymphoma? The definition of the word or special term fatigue and lethargy. [26] Laboratory study on hepatitis
“primary” is the key point to answer the question. The virus infection and serum l-lactate dehydrogenase
authors of this paper consulted several dictionaries and provides reference to treatment rather than diagnosis
encyclopedias including online medical dictionaries, of lymphoma. Serum levels of α-fetoprotein and other
especially the National Institutes of Health’s Web site, common tumor markers are usually normal. [31,32]
i.e. MedlinePlus, produced by the National Library of Imaging modalities are very important tools for
Medicine. The definition of the word “PRIMARY” detecting liver tumors and lymphoma, although the
[24]
can be summarized in two explanations. The first one majority of the diagnoses of liver lymphoma are
is general: first in order of time, place, development, established afterwards which is quite different from
or importance. However the second is medical: not the imaging diagnosis of mediastinal or retroperitoneal
derived from any other source or cause, especially the lymphoma. [33]
original condition or a group of symptoms in disease
processes, such as a primary infection or a primary Image modalities
tumor, arising spontaneously (idiopathic, efforts to find For evaluating primary liver tumors, currently useful
the original tumor have failed), being an initial tumor or image modalities include ultrasound, computed
74 Hepatoma Research ¦ Volume 3 ¦ May 09, 2017